Case Report: Two Newly Diagnosed Patients With KBG Syndrome-Two Different Molecular Changes.

Mutations or deletions of ANKRD11 gene are responsible for the symptoms of KBG syndrome. The KBG syndrome is a rare genetic disorder which is inherited in an autosomal dominant manner. Affected patients usually have characteristic facial features, macrodontia of the upper central incisors, hand abnormalities, developmental delay and short stature. In the present article we would like to report a clinical and molecular case study of two patients affected by KBG syndrome. The diagnosis of the first patient was confirmed by the identification of the novel pathogenic variant in ANKRD11 gene by next-generation sequencing. The second patient was diagnosed after the detection of a 16q24.2q24.3 deletion encompassing the ANKRD11 gene microarray.

Clinical factors in relapses of Wilms' tumor--results for the Polish Pediatric Solid Tumors Study Group.

BACKGROUND: The risk factors responsible for recurrences of Wilms' tumor (nephroblastoma) are still under discussion. The aim of the study was to analyze the relationship between relapses of Wilms' tumor and the patients' clinical history. MATERIAL AND METHODS: Clinical data from children registered in the Polish Pediatric Solid Tumors Study Group were analyzed. The clinical stages (CS), pathology variants (high risk: HR, intermediate risk: INT, and low risk: LOW) and chemotherapy regimens were correlated with the outcomes. RESULTS: Recurrences developed in 34 out of 288 (11.8%) patients with Wilms' tumor treated in accordance with International Society for Pediatric Oncology 2001 (SIOP 2001) protocols. Of these 34 patients, 11 initially had CS I, seven were at CS II, four were at CS III, 11 were at CS IV and one had CS V. There were eight patients with second recurrences; of these, seven were in the INT risk group and one in the high histological risk group. There was no correlation between age (p=0.256) or gender (p=0.538) and the risk of tumor recurrence. In the study group, seven out of 10 patients with local recurrences are alive; as are 13 out of 22 patients with distant recurrences (p=0.703). Those who died due to disease progression comprised six out of 26 patients with a first recurrence (four HR, two INT), and seven out of eight with a second recurrence (one HR, six INT). CONCLUSIONS: The prognosis after relapse in initially metastatic patients did not differ from that in patients who had primarily localized disease. The pathology variants probably had more significance.

Factors predicting survival in childhood malignant and intermediate vascular tumors : retrospective analysis of the Polish and German cooperative paediatric soft tissue sarcoma study groups and review of the literature.

BACKGROUND: The rarity of malignant and intermediate vascular tumors in children means that little is known about their clinical course, optimal treatment, and variables predicting survival. METHODS: A total of 32 children with malignant vascular tumors (14 angiosarcomas [AS], 5 epithelioid hemangioendotheliomas, and 13 intermediate vascular tumors, including other hemangioendotheliomas plus adult-type hemangiopericytomas), registered in the German and Polish Paediatric Soft Tissue Sarcomas Study Groups, were treated following the Cooperative Weichteilsarkom Studiengruppe (CWS)-81, -86, -91, and -96 protocols. RESULTS: Male sex, AS histology, tumor size >5 cm, and T2 invasiveness were independent predictors of inferior 5-year overall survival, while AS histology and T2 invasiveness were predictors of inferior 5-year event-free survival. AS histology was the most important negative prognostic factor for overall survival and event-free survival. Completeness of primary tumor excision was a good prognostic factor for survival in univariate, but not multivariate, analysis. Local therapy (radiotherapy and delayed surgery) were provided to the minority of patients (28% and 38%, respectively) late in the course of disease (after a mean of 9 and 6 months, respectively) and did not prevent local relapses. Response to systemic treatment was poor (44%) and did not prevent local and distant relapses. CONCLUSIONS: The clinical course and outcome in childhood epithelioid HE seems to be similar to intravascular tumors and less aggressive than AS. RTX and delayed surgery should be performed more frequently and earlier in the disease course. An urgent need for modification of systemic therapy is needed because of the development of many metastatic and/or combined relapses and poor response to classic chemotherapy. The problem of effective therapy for childhood AS is the most appaling: 13 of 14 patients died of progression despite multimodal treatment.

[Treatment results of children with neuroblastoma: report of Polish Pediatric Solid Tumor Group]. / Wyniki leczenia dzieci ze zwojakiem zarodkowym wspólczulnym: wstepny raport Polskiej Pediatrycznej Grupy Guzów Litych.

Approximately 60 children aged 0-18 years are diagnosed of NBL each year in Poland. About 60% of all patients suffering from NBL have a chance for durable cure. Unfortunately the prognosis for patients within the high-risk group accounting for more than 50% of all NBL patients remains poor despite the introduction of more intensive chemotherapy regimens with radical surgery procedures and megachemotherapy with subsequent stem cell transplantation. Only one third of patients in this group can be cured. To improve the treatment results of the high-risk patient group and to decrease the rate of therapy related side effects current European treatment protocols have been introduced systematically in Poland. In February 2009 information about 389 patients (age 0.1-16.5 years) diagnosed between 2001 and 2008 were obtained. Results of therapy of 319 patients who started treatment from 2001 to 2007 were analyzed. Between 104 infants and 215 children over 1 year of age, stage 4 of disease was found in 25% and 54.5%, respectively. In this period additionally to European treatment protocols, two another protocols were used. Satisfactory treatment results were obtained in 104 infants (5-year event free survival /EFS/=82.6%), irrespective of the type of treatment protocol. Over 5-year EFS for children over 1 year of age in 1, 2 and 3 stage of disease was: 100%, 86.3% and 64.5%, respectively. On the contrary, 107 patients with 4 stage of disease achieved the 5-year EFS of 27% only. Treatment results obtained in patients treated according to the European HR-NBL-1/ESIOP protocol were better than for patients treated according to other treatment protocols (5-year EFS: 31.1% and 16.4%, respectively), but difference between these groups was not significant. Between 2001 and 2007 data reporting increased to 81% from 19% noted earlier. Unfortunately, results of treatment for children over 1 year of age remain still unsatisfactory. That is why there is a need of improvement of modern, unified treatment realization as well as better data reporting. For realization of these aims adequate financial support is essential.

Retrospective multi-institutional study on hemangiopericytoma in Polish children.

BACKGROUND: Pediatric hemangiopericytoma (HPC) is an extremely rare vascular tumor with little data available on its clinical course and management. METHODS: Fourteen children with HPC registered in Polish Pediatric Rare Tumors and Polish Pediatric Soft-tissue Sarcomas Studies between 1992 and 2002 are reported. RESULTS: Seven patients (F/M: 5/2, age 2-10 months) had infantile HPC, four of whom had primary tumors affecting superficial tissues of the trunk and upper limbs. No child had initial nodal or organ metastases. Primary excision (PE) was performed only in three patients with superficial lesions. They received no supplemental treatment. The remaining four children responded well to chemotherapy (CHT), entering complete remission after CHT alone (n= 2) or after delayed resection (n= 2). No relapses occurred and all patients were alive 48-146 months after treatment. Seven patients (F/M: 2/5, age 3.2-16.5 years) had adult-type HPC, five of whom had tumors localized in superficial tissues of the lower limbs or head/neck. All patients presented with locally advanced disease. PE was performed in five children (complete in four); all patients were alive at follow up of 40-127 months. Three patients died of recurrence after incomplete PE despite supplemental chemo- and radiotherapy. CONCLUSIONS: Complete surgical excision remains the mainstay of treatment for both HPC types. In unresectable adult-type HPC adjuvant chemo- and radiotherapy should be administered in macro- and microscopic tumor residues, but the prognosis is poor despite supplemental treatment. High chemo-responsiveness of infantile-type HPC produces a favorable outcome even in cases of unresectable, life-threatening tumors.

[Soft tissue sarcomas of the parameningeal region in children--own observations]. / Miesaki tkanek miekkich okolicy okolooponowej u dzieci--obserwacje wlasne.

UNLABELLED: Soft tissue sarcomas (STS) non-Hodgkin's lymphomas and less frequently nasopharyngeal carcinomas are the most common malignancies located in the parameningeal region in children. AIM: To assess diagnostic and therapeutic problems in children with parameningeal STS treated in the Departments of Paediatric Oncology in Gdansk and Lublin between 1992 and 2006. MATERIAL AND METHODS: The study includes 17 patients with parameningeal STS; mean age of patients was 5.6 years. In one boy an undifferentiated STS was diagnosed 7 years after treatment of retinoblastoma. RESULTS: Initial symptoms lasted from 2 weeks to 24 months, mean 5.5 months. Symptoms associated with parameningeal location of the tumour (snoring, breathing through the mouth, epistaxis, chronic purulent rhinitis, dysphagia and earache) predominated and were treated initially as upper respiratory tract infections. All analysed patients presented with highly advanced stages of STS. Oncological treatment was conducted according to the schemes approved by the Polish Paediatric Solid Tumours Study Group. Good response to therapy was stated only in 24% children with STS. These patients (all with embryonal subtype) entered complete remission after standard I line therapy. 13 children required more aggressive II line treatment because of poor response to therapy (NR - 5 children) or relapse (8 children). Seven of the analysed patients (41%) are in lasting complete remission, from 32 months to 13 years 2 months (mean 5 years) after therapy discontinuation. In four children (23%) persistent complications of oncological treatment occurred, including postradiation defect of the orbital bulb, postsurgical facial nerve palsy and cranio-nasal fistula complicated with pneumocephaly. A patient with STS of maxillary sinus developed a second neoplasm 2 years after first therapy. This was a glioblastoma multiforme located in the left parietal lobe (outside the radiation field). At present, the boy is in complete remission nearly 4.5 years after treatment for the second tumour. Ten patients died, all in the phase of disease progression. In two of them myelosupressive, gastrotoxic and infectious complications of antitumour therapy were the direct cause of death. CONCLUSIONS: 1. Non-specific initial symptoms of soft tissue sarcomas located in parameningeal region in children suggesting inflammatory process result in diagnostic dilemmas and proper diagnosis delay. 2. Because complete resection of the parameningeal STS is unfeasible, the prognosis is poor in spite of aggressive chemo- and radiotherapy. 3. Complex therapy carries a risk of severe complications, thus it should be conducted in highly specialized oncological centres.

[Incidence of childhood leukemia in the Lublin region of Poland in 1988-2000]. / Wystepowanie bialaczek u dzieci i mlodziezy na Lubelszczyznie w Latach 1988-2000.

UNLABELLED: Purpose of the work was the analysis of the number and structure of leukemia in children living in the Lublin Region of Poland. The study included the population of children aged 0-17 in the years 1988-2000. Among leukemias, according to the International Classification of Childhood Cancers, were counted: lymphoid leukemia, acute non-lymphocytic leukemia, chronic myeloid leukemia, other specified and unspecified leukemias. Number of cases and incidence for the whole group as well as sex, age and descending (rural and urban) distribution of leukemias were calculated. RESULTS: 244 cases of leukemia were reported (152 boys--62.3% and 92 girls--37.7%). The most of children were from urban regions (139-57%). The mean morbidity rate was 29.9 per million (among boys--36.4/mln, girls--23.1/min). The highest morbidity was observed in years 1989-1990. After 1992 year vehemently decrease of morbidity was noted. Incidence fall with the age, the higher was found in the 0-4 years group (58.7/mln). The decreasing incidence was noted in following years of observation only among the youngest children, while the incidence in children 5-17 years was stable. The higher morbidity was observed among children living in urban regions than in rural ones (35.7/mln vs 24.6/mln) and the incidence in urban region decreased. CONCLUSIONS: In the Lublin Region lower percentage of leukemia was observed compared to the values determined for the country. Incidence of leukemia was falling, but the analysis in age-groups proved, that it was caused by the decreasing incidence in children under 5 year. The higher morbidity was noted in urban children than in rural ones.

[Comparative analysis of incidence of childhood cancers in four former Provinces of Lublin Region of Poland in 1988-1998]. / Analiza porównawcza zachorowalnosci na nowotwory zlosliwe wsród dzieci i mlodziezy w czterech województwach makroregionu lubelskiego w latach 1988-1998.

UNLABELLED: The growth of a neoplasm results from interactions of various carcinogenic factors, also environmental ones, so incidence of cancers depends on the place of living. Purpose of the work was the comparative analysis of the number and morbidity of childhood cancers in children living in four former Provinces of Lublin Region of Poland. MATERIALS AND METHODS: The study included the population of children and young adolescents, aged 0-17, living in the area of four former Provinces--Biala Podlaska, Chelm, Lublin and Zamosc--of the Lublin Region in the years 1988-1998. The analysis determined the number and incidence (Incidence Rate, IR) of all childhood cancers, the parameters were calculated for the whole population according to sex, age on diagnosis and the place of living-urban or rural regions. RESULTS: Between 1988 and 1998 a total of 856 cases of childhood cancers were noted in Lublin region: 154 cases (IR--141.8 per million) in Biala Podlaska Province, 105 cases (IR--122.0) in Chelm, 403 cases (IR--121.5) in Lublin and 194 cases (IR--115.8) in Zamosc. In the 11-year-period of examination the highest incidence was stated in Biala Podlaska Province, but not statistic substantially. Higher incidence revealed among boys (except Zamosc Province). Incidence was higher in urban areas than in rural ones in Chelm and Zamosc Provinces and the same in urban and rural areas in Biala Podlaska and Lublin Provinces. CONCLUSIONS: 1) The highest incidence of childhood cancers in the Lublin Region was stated in Biala Podlaska Province (it was not statistic substantially), 2) only in Chelm and Zamosc Provinces the incidence were higher in urban areas than in rural ones.

[Malignant neoplasms localised in the parameningeal region in children treated at two Polish oncological centres-diagnostic and therapeutic dilemmas]. / Nowotwory zlosliwe o lokalizacji okolooponowej u dzieci w materiale dwóch osrodków onkologicznych--trudnosci diagnostyczne i terapeutyczne.

UNLABELLED: Malignant neoplasms localized in the parameningeal region include mainly soft tissue sarcomas (MTM), non-Hodgkin s lymphomas (NHL-B) and, less frequently, nasopharyngeal carcinomas. The aim of the study was to analyze diagnostic and therapeutic problems in children with parameniingeal neoplasms treated in Departments of Paediatric Oncology in Gdansk and Lublin between 1992 and 2004. MATERIAL AND METHODS: The study includes 32 patients (M/F: 23/9), aged 2 to 17 years, mean 6,3 years. In 17 children MTM was diagnosed: in nine NHL-B-cell and in six--nasopharyngeal carcinoma (lymphoepithelioma). The diagnosis of NHL-B and undifferentiated MTM were made in two children treated previously for NHL-nonB and retinoblastoma. Two cases of NHL appeared in a girl with ataxia-teleangiectasia syndrome. RESULTS: Initial symptoms lasted from 2 weeks to 24 months, mean 4,5 months for the whole group. In NHL patients mean period ofsymptoms was 4,5 weeks, in MTM- 5,5 months and in lynmphoepithelioma--7 months. Symptoms associated with the tumours localisation (snoring, breathing through the mouth, epistaxis, chronic purulent rhinitis, dysphagia and earache) predominated and were treated initially as upper respiratory tract infections. Cervical lymph nodes enlargement was observed in 30% children with MTM and 83% with lymphoepithelioma. Most of patients presented with highly advanced stages of neoplasms. MTM and NHL-B treatment was conducted according to the protocol approved by the Polish Paediatric Solid Tumours and Leukaemia/Lymphoma Studies Group. In patients with lymphoepithelioma different treatment schemes were administered, including chemo- and radiotherapy. Good response to therapy was found in 13/32 patients (41%). The group included 24% children with MTM (all with embryonic subtype), 56% with NHL-B and 67% with lymphoepithelioma. All these patients attained complete remission after standard line I therapy. But 13 children with MTM, four with NHL-B and two with lymphoepithelioma required more aggressive line II treatment because ofpoor response to therapy (NR) or relapse. Finally, 20 of 32 followed-up patients (62,5%) are in durable complete remission from 10 months to 11 years 4 months (mean 4 years) after therapy discontinuation. This group consists of all nine patients with NHL-B, 67% children with lymphoepithelioma and 41% with MTM. In six children (30%) persistent complications of oncological treatment occurred, including: hypoacusia, postradiation defect of the eye ball, postsurgical facial nerve palsy and cranio-nasal fistula complicated with pneumocephaly. A patient with MTM of maxillary sinus developed a second neoplasm 2 years after first therapy. This was glioblastoma multiforme located in the left parietal lobe (outside the radiation field). At present, the boy is in complete remission 2,5 years after treatment for the second tumour Among 32 children with parameningeal neoplasms 11 patients died (nine with MTM and two with lymphoepithelioma), all in the phase of disease progression (five NR and six after relapse). In two of them (with MTM) the direct cause of death was myelosupressive, gastrotoxic and infectious complications of antitumour therapy. One child still undergoes treatment for MTM relapse.

Incidence and clinical characteristics of second malignant neoplasms in children: a multicenter study of a polish pediatric leukemia/lymphoma group.

BACKGROUND: The development of second malignant neoplasms (SMNs) in patients receiving chemotherapy and radiation therapy for primary cancers is one of the limitations to the quality and length of survival. The present study was undertaken to examine various characteristics of children who developed SMNs following successful therapy for primary leukemia or Hodgkin's disease (HD). MATERIAL/METHODS: A total of 3252 children with various forms of leukemia and 849 children with HD treated between, 1970-1997 at 7 pediatric centers of the Polish Pediatric Hematology/Oncology Group and subsequently followed-up entered the study. A second malignancy was diagnosed in 36 of these children. RESULTS: Of the 3252 patients diagnosed as having acute leukemia during this period, 16 developed SMNs (estimated frequency 0.49%). SMNs developed in 20 of the 849 children treated for HD (2.36%). The most frequent SMNs were soft tissue sarcoma and thyroid carcinomas, mainly following Hodgkin's disease. Other tumors occurred at about the same frequencies in both groups. The interval from the end of initial treatment to diagnosis of an SMN ranged from 2 years 7 months to 17 years 6 months, with a median of 7 yrs 4 mo. for acute lymphoblastic leukemia (ALL) patients and 10 years for children with HD. The estimated accumulated risk of SMN following acute leukemia is 0.95% at 15 years and, for HD, 5.1% at 20 yrs and 7% at 25 yrs. CONCLUSIONS: Children who have been successfully treated for one cancer have a higher than expected incidence of additional tumors.

Incidence of childhood cancers in rural areas of the Lublin Region in 1988-2000.

UNLABELLED: The purpose of the work was the analysis of the number and structure of new neoplasm and morbidity in children living in rural areas of the Lublin Region of Poland. METHODS: the study included the population of children aged 0-17 in the years 1988-2000. The tumours were divided according to the International Classification of Childhood Cancers. The analysis determined the incidence of all tumours and individual types and the rates of percentage structure and incidence. These parameters were calculated for the whole population according to sex and age. RESULTS: 430 cases of childhood cancers were reported. Boys were 53.7%. The most frequent was leukemia (24.4%), neoplasm of CNS (19.5%) and lymphomas (16%). The mean morbidity rate was 100,8 per million (among boys--105.6, girls--95.8). CONCLUSIONS: I. The patient's place of residence is one of the factors affecting the structure of cancers. 2. Higher percentage of lymphomas and CNS tumours and lower percentage of leucaemias and tumours of the sympathetic nervous system and bones was observed in the Lublin Region compared to the values determined for the whole country. 3. The population examined showed lower malignant tumour incidence than that in the whole country as well as increased lymphoma incidence.

The radiologic diagnosis of invasive fungal infections in neutropenic children.

Fungal infections have substantially increased in incidence over the past two decades, especially among patients with cancer who have received high doses of chemotherapy. Diagnosis is based on clinical observations, radiological and laboratory methods and molecular biological techniques. Early diagnosis is important and increases overall response rates, so high-risk patients should be treated empirically, based on the clinical picture and radiologic findings alone. The aim of the study was the analysis of the early clinical picture and radiologic findings of systemic fungal infections. The subjects of this study were patients with childhood cancers and fungal systemic infections. The early symptoms of fungal infection and the radiologic findings were analyzed. The systemic fungal infections were recognized in eight children, treated due to ALL--in four patients, AML--in two and NHL--in two. In all, manifestations of fungal infection were observed during bone marrow suppression secondary to chemotherapy treatment of neoplasm. In all, X-ray examination was normal. Radiological pulmonary changes were found in CT scans in seven patients, in one MRI showed marked abnormnalities of the brain. All patients received intensive antifungal drugs, six patients had a complete or partial response and they were able to continue chemotherapy for treatment of neoplasm. The regression of pulmonary nodules was observed-in the control CT scans. Progression of fungal infection was noticed in two patients, these children died, Conclusions: The early diagnosis of fungal infection in neutropenic patients is important and increases overall response rates. The pulmonary fungal infection should be considered in the differential diagnosis of solitary or multiple pulmonary nodules, particularly in immunocompromised patients.

Outcome of children with idiopathic thrombocytopenic purpura, based on original material.

Idiopathic or immune thrombocytopenia purpura (ITP) is a common hematologic disease in children. ITP is subdivided in an acute and chronic form based solely on the duration of thrombocytopenia. The aim of the study was to analyse the course of disease and outcome of children with acute and chronic ITP. The study enrolled 52 patients with ITP, who were treated in the Department of Pediatric Hematology and Oncology in Lublin from 1998 to 2002. Of the 52 patients with ITP there were 28 girls and 24 boys aged from 12 months to 17 years. Out of the 52 patients, 3 patients had spontaneous recovery, 33 were treated with steroids, one with immunoglobulin, others with combined therapy. Good response to treatment was found in 40 patients. Nine children developed chronic ITP. Out of 9 patients, 6 had splenectomy with good result and 3 are only observed. The benefits of splenectomy in markedly symptomatic chronic ITP far outweigh the risks. The majority of patients with acute ITP had good response after corticosteroids therapy.

[Malignant vascular tumours in children -- report from the Polish Pediatric Rare Tumors Study]. / Charakterystyka kliniczna naczyniopochodnych nowotworów zlosliwych u dzieci -- raport Programu Guzów Rzadkich PPGGL.

UNLABELLED: Malignant vascular tumours represent a rare group of neoplasms, usually treated according to protocols for soft tissue sarcomas. THE AIM OF THE STUDY: To assess the clinical characteristics, disease course and outcome in Polish children with malignant vascular neoplasms. MATERIAL AND METHODS: The Polish Paediatric Rare Tumours Study retrospectively analysed multicentre data on 32 children with vascular tumours, registered between 1992 and 2002. On the basis of the histological type of the neoplasm these patients have been divided into three groups: group I -- 10 patients with angiosarcoma (ASA), group II -- 7 children with haemangioendothelioma (HE) and group III- 15 patients with haemangiopericytoma (HP), of both infantile (7 children) and adult-types (8 patients). RESULTS: Group I: 5 patients presented with local, 3 with systemic and 2 with regional disease. Primary complete resection (PRC) was performed in 7 patients, not resulting in local control in any of them. Radiotherapy (RTX) was administered in 5 patients, chemotherapy (CHT) in all. 7 patients relapsed, 3 never entered clinical remission. 9 of the 10 patients of group I, died of disease progression. Group II: PRC was performed in 5 patients and led to local control in 2. Adjuvant RTX was used in 2 and CHT in 4 patients. 2 children relapsed and died of the disease. Infantile HP: PRC was performed in 3 children, remaining 4 patients received adjuvant CHT All children entered complete remission and are disease-free. Adult-type HP: PRC was performed in 5 patients, resulting in local control in 4. Five children were given adjuvant RTX and six CHT Three patients relapsed and died of disease progression. CONCLUSIONS: The effectiveness of primary complete resection in all groups was doubtful. The high rate of metastatic relapses suggests that the currently given systemic therapy is not satisfactory. The only tumour with excellent prognosis was infantile type HP (all patients are alive and free of disease). Adequate treatment for children with angiosarcoma remains still unknown -- 9 of 10 patients died of disease progression. Prognosis in patients with haemangioendothelioma is intermediate, however the role of immunotherapy should be further investigated.

[Central nervous system metastases in children with solid tumours]. / Przerzuty do osrodkowego ukladu nerwowego w guzach litych u dzieci.

BACKGROUND: Central nervous system (CNS) metastases occur in 20-30% of adult patients with systemic cancers. but they rarely occur in children with solid tumours. AIM: clinical and prognostic characteristics of CNS recurrence in children treated for solid tumours were analysed. PATIENTS AND METHODS: The retrospective study enrolled 218 children treated for solid tumours in the Department of Paediatric Haematology and Oncology, Lublin Medical Academy, from January 1992 to December 2002. The diagnosis in this group was as follow: soft tissue sarcomas -- 51 patients, bone tumours -- 50. Wilms' tumour -- 48, neuroblastoma (NBL) -- 36, germ cell tumours -- 33: Children with primary CNS tumours, retinoblastoma, lymphoma and rare tumours were not analysed. RESULTS: CNS metastases were diagnosed in five children (2.3%) - (2 boys. 3 girls; aged 2,5 to 17 years). Two of them were treated due to Wilms' tumour, one -- NBL, one -- teratoma malignum, one -- leiomyosarcoma. None of the children with bone tumours had CNS metastases. Diagnosis of CNS metastases was confirmed by imaging studies (CT, MRI). The median time from initial diagnosis to the detection of CNS metastases was 14 months. Two children underwent surgical resection of solitary metastases. One of them was also irradiated and received chemotherapy and only this child is alive and achieved complete remission. Other children died, with median period of 32 days. CONCLUSIONS: CNS metastases may occur in children with the recurrence of primary neoplastic disease. The prognosis is grave.

[Rare tumours--are they really rare in the Polish children population?]. / Nowotwory rzadkie--czy rzeczywiscie rzadkie w populacji polskich dzieci?

Clear statement that pediatric neoplasms are really rare is not easy. Thus the incidence of rare tumours in children has not been defined so far. The paper efforts to assess the topic of rare tumours of childhood in the Polish population. Following two categories are proposed: tumours typical for adults, but possible in children (neoplasms of epithelial origin--mainly carcinomas, melanomas, carcinoids) and paediatric tumours consisting less than 10% of cases in corresponding clinical groups according to the ICCC classification. Data on 317 patients aged 0-18 years treated in centres associated in the Polish Paediatric Group for Solid Tumours (PPGST) were analysed. Classical adult malignancies were registered in 130 patients: carcinomas in 90 (mean age 12.6 +/- 4.5 years), melanomas in 25 (mean age 9.4 +/- 4.9) and carcinoids in 9 (mean age 14.5 +/- 1.2 years). Non epithelial neoplasms were registered in 187 patients (mean age 10.4 +/- 5.5). That group included rare tumours of soft tissue, CNS, bones and other organs. Treatments of certain groups were specified by separate therapeutic protocols within PPGST. Rare malignancies of adult-type among children under 18 years of age in Poland comprised 1.5% of all pediatric neoplasms. The incidence of adult-type neoplasms increased with age until 14 years. In patients over 15 years of age the number of registered cases decreased. It may suggest a first peak of incidence in early adolescence or an underestimation of number of patients with carcinoma aged over 15 years. In the analyzed group, the mean age of patients with carcinomas and other epithelial and unspecified tumours significantly exceeded the age of children with rare neoplasms of non-epithelial origin (12.1 +/- 4.7 vs 10.4 +/- 5.5 years; p<0.05). A very young age at diagnosis of malignant melanomas (mean 9.4 years) and numerous cases of carcinomas affecting the digestive tract (n=24; 27% of all carcinomas), especially those located in colorectal region (n=10), seem surprising. The preliminary analysis of the collected data on rare neoplasms in Poland encourage to undertake a prospective study, meant to link the epidemiology and characteristics of rare epithelial tumours in childhood with diagnostic and therapeutic suggestions for these types that are not coordinated within Polish Paediatric Group of Solid Tumours.

[Second neoplasm in a 13-year-old boy complicated by Crohn's disease. Case report]. / Drugi nowotwór powiklany choroba Crohna u 13-letniego chlopca. Opis przypadku.

UNLABELLED: Genetic abnormalities and immune system disorders resulting from them are the causes of neoplastic diseases as well as inflammatory bowel disease, including Crohn's disease. However, the rearrangement of genes may not only be the cause of neoplasms but also the result of oncolytic treatment used. One of the late treatment-related complications of neoplastic diseases is the development of a second neoplasm and possible disorders belonging to the group of inflammatory bowel diseases, for example Crohn's disease. The paper presents the case of a 13-year-old boy (K.G.) treated for a second neoplasm, complicated by Crohn's disease. The patient was diagnosed with acute lymphoblastic leukaemia -- middle risk group (ALL-MRG) at the age of 3, treated according to the BFM 87 Protocol (dexamethasone, prednisone, vincristine, daunoribicin, asparaginase, cyclophosphamide, cytarabine, mercaptopurine, methotrexate, thioguanine, doxorubicin), and complete remission was achieved. Eight years after the first line treatment the boy was diagnosed with the second neoplastic process -- pre-B acute lymphoblastic leukaemia (ALL-L2 pre-B common +). The ALLIC 2002 Protocol (dexamethasone, prednisone, vincristine, daunoribicin, asparaginase, cyclophosphamide, cytarabine, mercaptopurine. methotrexate, thioguanine, doxorubicin) was started. On day 71 of the therapy, during aplasia of blood marrow following chemotherapy, inflammation of the caecum was diagnosed and metronidazole was introduced. Once blood counts improved, the complaints decreased and with continued chemotherapy of Protocol M (mercaptopurine, methotrexate), completely subsided. The symptoms reappeared during the 2-week break before Protoco II. The physical examination revealed the presence of a hard, painless tumour (3 x 10 cm) in the right iliac fossa. Colonoscopy showed a cauliflower-like formation within the Mauchin's valve projecting to the caecum lumen. The histopathological evaluation of the sections demonstrated a typical picture of Crohn's disease. Crohn's disease of the ileum was diagnosed, the treatment with dexamethazone (according to Protocol II) was instituted which led to complete regression of the iliac lesions. Steroid therapy (prednisone) continued until the treatment supporting the remission according to Protocol ALLIC 2002 (mercaptopurine, methotrexate orally) was initiated. CONCLUSION: The differential diagnosis of complications accompanying neoplasms should consider the inflammatory bowel diseases; their atypical course may be masked by the treatment of the underlying disease.

[Results of treatment for osteosarcoma in children and youth]. / Analiza wyników leczenia miesaka kosciopochodnego u dzieci i mlodiezy w materiale wlasnym.

Bone tumours comprise about 5% of childhood neoplasms. Osteosarcoma is the most common sub-type and constitutes 60 % of all bone cancers. Incidence is similar in girls and boys and is increasing steeply with age. The most common localization of this tumour is long bones of the legs. Treatment including chemotherapy and surgery has led to an increase of the percentage of non-mutilating recovery. We present treatment results of 14 children with osteosarcoma.

[Incidence of childhood solid tumours in Lublin region from 1988 to 2000]. / Wystepowanie nowotworów litych wieku dzieciecego na Lubelszczyznie w latach 1988-2000.

The purpose of this study was the analysis of number and structure of new solid tumours and tumour morbidity among children 0-17 years old, in the Lublin Region of Poland, from 1988 to 2000. The analysis was based on sex, age and place of domicile (urban/rural region). During this time in the Lublin Region 344 cases of childhood solid tumours were reported; it was 36.4% of all childhood cancers diagnosed in this region. The structure of the disease was related to sex, age and the place of living. The mean morbidity rate was 42.2 per 1 mln (among boys - 42.2, girls - 42.4). The highest incidence rate was observed among children 0-4 years old (78.1 per 1 million). Higher morbidity was noted among patients living in urban regions (47.3 vs 37.6 / million).

Primary chest tumours in children.

Primary chest tumours in children are rare and appear in 3% of cases, 83% of which are malignant. Early diagnosis is very difficult because of delayed symptoms and highly advanced stage. Symptoms of chest tumours depend on the tumour mass, localization and time of progression. These tumours can cause cough, dyspnoea, Horner's syndrome and superior vena cava syndrome. The purpose of this study was to analyze the kind of chest tumours, clinical symptoms before diagnosis, their duration and results of treatment.